I've been meaning to update on this for a while. I just haven't been able to get it done.
Dr. Francomano has very thorough appointments, and she even sends you home with a summary of everything discussed during your visit. So, I just have to go through my 8-page "Initial Consultation Summary" and decide what information is the most important. I'll do my best below. Afterward, I'll include any thoughts on the appointment.
First, there was a very complete medical history taken. I'll skip over that, since that information is all available at my website, which is now archived (http://zippergirl28.tripod.com).
There is then a list of diagnoses from my history:
*Chiari 1 Malformation, decompressed 2002
*Dysautonomia, POTS, diagnosed 2003
*Cranio-cervical instability/cranial settling, status/post fusion 12/2003
*Occult tethered cord, status/post untethering 6/2008
*GERD
*Paipilledema
*Osteopenia, diagnosed 3/2008
*Hyperparathyroidism
*History of Vitamin D deficiency
*IBS
*Scoliosis
*Restless Leg Syndrome/Periodic Limb Movement Disorder
*Hypovolemia
*Bicuspid Aortic valve
*Aortic Regurgitation
Surgical History
*Tonsillectomy, 3/2002
*Posterior fossa decompression, craniectomy, C-1 laminectomy, 7/2002
*Invasive cervical traction, 11/2003
*Cranio-cervical extraction fusion, skull to C-4, 12/2003
*Cholecystectomy, 4/2008
*Section of Filum Terminale, 6/2008
*Repeat invasive cervical traction, 6/2008
Hospitalizations
*1/16/03-1/25/03: severe drop attacks and blackouts, diagnosed with POTS
Known Allergies
*Compazine: dystonic reaction
*Wellbutrin: seizure
*Sulfa drugs: severe gastrointestinal distress
*Opiates: rash, severe pruritis
*Adhesives: skin deterioration
*Sensitivity to many medications, especially antibiotics and narcotics
Review of Systems
Frequent fevers
Difficulty falling asleep
Difficulty staying asleep
Chronic fatigue
Nearsightedness
Ringing in the ears
Swallowing difficulties
Shortness of breath
Ches pain
Racing heart beat
Light-headedness
Fainting
Heart murmur
Exercise intolerance
Nausea
Abdominal pain
Digestive problems
Urinary urgency
Urinary frequency
Easy bruising
Temperature instability
Migraine headaches
Non-migraine headaches
Tremors
Numbness
Frequent falls
Brain fog
Loose joints
Joint pain
Joint subluxations
Muscle pain
Scoliosis
Vertigo
Palpitations
Myoclonic jerking
Next was the review of my family history. I list a couple of the key points here:
A three-generational family history was obtained. Pertinent findings include:
*Joint pain and history of two shoulder dislocations in one family member
*Knee subluxations and joint pain in one family member
*Joint laxity in one family member
*Joint laxity and history of dislocations in one family member
*Knee subluxations in one family member
*Intra-cranial hypotension, spinal cord leak, hip subluxations, and joint laxity in one family member
*Degenerative disc disease in one family member
*Osteoporosis, scoliosis, arthritis, and loose knees in one family member
*Migraines, early-onset osteo-arthritis, weak ankles in one family member
*loose ankles, runner's knee, and tendonitis in wrist in one family member
*Atrial septal defect, arthritis, and degenerative disc disease in one family member
Pertinent Findings from the Physical Examination
Height: 64" (I shrunk again!)
Facial Features:
Grey sclerae (The whites of my eyes are not white when you shine a light in them; they're grey, because the connective tissue is very thin.)
History of dental crowding
Palate: High, not narrow on examination, but she is status post palatal expansion
Uvula: asymmetric
Other: widely dilated pupils, symmetric, reactive to light (must have been a good day)
Neuromuscular
Abnormal: absent proprioception, marked dysmetria on finger to nose testing, difficulty with rapid alternating movements. Reflexes are 2-3+ and symmetric. Downbeat nystagmus noted. No clonus, no Babinski. (Basically, this means that I have a number of strange neurological responses.)
Musculoskeletal
Downsloping ribs present (My ribs go down lower than they should, so there's a short distance between my rib bones and my hip bones.)
Beighton Score
Passive dorsiflexion of 5th MCP >90 deg. R and L (My pinkie finger can be bent back beyond 90 degrees, barely. That gets me 2 points.)
Thumb can touch forearm R and L (I can do this on both sides, getting me points 3 and 4.)
Elbow hyperextension beyond 10 deg. R and L (She actually measured. Every other doctor just "eyeballed" it. Both elbows did bend backward more than 10 degrees. Points 5 and 6)
Knee hyperextension beyond 10 deg. R and L (Both were quite bendy. Points 7 and 8)
Can touch palms on ground with knees extended. No (I always could do this one, but haven't been able to since my detethering, for some reason. So, my Beighton Score is officially on 8 out of 9. Much higher than other doctors have measured it.) Measurements to follow.
More hypermobility
Able to touch tongue to tip of nose
Shoulder Hypermobility: Hyperextension on both sides
Elbow Hypermobility:
Left: 15 degrees hyperextension
Right: 11 degrees hyperextension
Hip Hypermobility: Hyperextension on both sides
Knee Hypermobility:
Left: 20 degrees hyperextension
Right: 25 degrees hyperextension
Small Joint Hypermobility
CMC joint hyperextension
MCP joint hyperextension
DIP joint hyperextension
(My hands really are not very flexible. You want to see crazy flexible hands, you should see Gustavo's. He doesn't have EDS. Just stretchy hands.)
Pes Planus present (I have flat feet.)
Piezogenic papules present (These little tiny bumps that appear on the inside of my heel when I stand.)
Scoliosis present
Straightening of the cervical lordosis present
Straightening of the thoracic kyphosis present
Lower thoracic lordosis present
Skin
Soft texture present.
Hyperextensibility of the skin present (I never thought I had this. But she stretched the skin on my hand and around my neck and I guess it stretched more than normal.)
Translucent skin present (We all knew this one...everyone whose ever tried to do my makeup!)
Scarring present: atrophic scars on both knees. Well healed surgical scars, cervical and lumbar.
Evidence of bruising
Other Findings
She is wearing a Vista collar. There is a II/VI systolic ejection murmur heard best at the left upper sternal border.
Impression
Based on the above, it is our impression that Ms. Richardson has joint hypermobility, soft, translucent, stretchy skin, and craniofacial features consistent with classical Ehlers Danlos Syndrome. Her bicuspid aortic valve and the atrial septal defect in her [family member] are of interest and may suggest a specific sub-type of heritable connective tissue that has been described recently.
The family history is remarkable for connective tissue findings on both sides of the family and it is possible that she has inherited one allele from her mother and another from her father.
Other notes
"EDS affects collagen. Collagen is a protein, which acts as a 'glue' in the body, adding strength and elasticity to connective tissue.
It is important to note that the range and severity of symptoms may range from person to person, even within the same family. Most types of EDS are inherited in an autosomal dominant manner. This means that if a parent has EDS, each of his or her children have a 50% chance of also having EDS.
(We also discussed the genetics of the trifecta. She agreed that it is much more unpredictable, and while research is being done, we may never be able to predict or prevent passing on the trifecta. She couldn't say much more about advising me in my decision, because we just don't know. She was aware of "trifecta families", in which both Chiari and EDS seem to be passed down at a much higher rate than they would on their own.)
Individuals with joint hypermobility often experience chronic muscle and joint pain. The age of onset may vary from adolescence to older adulthood. The pain can vary in the number of sites of the body that it affects, in duration, severity, and response to therapy. The severity of pain is generally greater than what is expected based on physical and radiologic examination. Sleep disturbances and chronic fatigue are often present among individuals with chronic pain. Affected individuals are often diagnosed with chronic fatigue syndrome, fibromyalgia, depression, and hypochondriasis prior to receiving a diagnosis of joint hypermobility. The cause of the pain is not entirely understood. However, it is thought that the pain experienced by individuals with joint hypermobility is related to myofascial and neuropathic pain. Myofascial pain is usually located around or between the joints and is often described as aching, throbbing, or stiff and may be caused by muscle spasms. Chronic joint instability may cause the myofascial pain, as the muscles attempt to respond to the joint instability. Neuropathic pain may be caused by nerve impingement (caused by subluxed vertebrae, herniated discs, osteoarthritis, or other subluxed joints) and is often described as electrical, burning, shooting, numb, tingling, or hot or cold discomfort." All quoted from Dr. Francomano
Recommendations
Aquatic physical therapy for muscle strengthening (however, she said to hold off for now, because my body is not ready at this point)
Myofascial trigger point workbook to help with myofascial pain
Lidoderm patches for pain
Actipatch for pain
Orthotics for flat feet (I wonder if I just get these at the store, or go to the orthotist?)
Diclofenac topical for joint pain
Epsom salt foot baths
Follow up with TCI as planned
And that was it. I really like her. I would say the only thing that disappointed me a bit were the treatment options. I didn't realize there was so little to do for EDS pain. I'm not sure where to find a myofascial release practitioner, and she prefers that it is a physiatrist, and I prefer that it is one covered by insurance. Either way, I'm not sure that right now is the best time to start a new therapy regimen.
Also, I had 8 questions I prepared for her, but she didn't really have any answers for me.
She had no ideas on recovering from surgery.
She had no ideas on finding a physical therapist or a myofascial release specialist.
She had no ideas on my episodes of reduced consciousness.
She had no ideas on maintaining bone density after discontinuing Forteo.
She had no suggestions on IBS other than fiber.
So, I guess I thought she would have more to recommend on those ideas, but I definitely should have focused more on EDS, but those really are related to EDS, mostly.
I'm still glad I went. I have a definitive diagnosis. I have another specialist on my side. And I believe I'll be able to count on her if a problem arises.
Thursday, February 25, 2010
Tuesday, February 23, 2010
A Very Big Day
After being diagnosed with osteopenia in March 2008, then continuing cranial settling in June 2008, I knew exactly what I needed to do. I needed to have my cranio-cervical fusion revised. I knew that if I could have my skull raised and re-fused into the sweet spot, I would have less pain, more energy, and a higher quality of life. Dr. Milhorat believed that was in my future, and I do, too. Before I could have that surgery, I would need to have my osteopenia corrected. The medication Forteo was recommended for me by my New York doctors.
Unfortunately, this would be a much bigger challenge than I anticipated. My first endocrinologist refused to treat me, because I was too young, and my osteopenia was not severe enough to require treatment. So, I went to a new endocrinologist. She insisted on trying a year of conservative treatment first. I had no choice. She was willing to work with me and my doctors in New York, so she was the best choice I had. The conservative treatment involved calcium supplementation and mega-doses of vitamin D. I ended up with too much vitamin D in my system, but I still wasn't absorbing enough calcium, based on the blood and urine tests. So, on October 26, 2009, she finally prescribed Forteo, which is one of the only medications known to actually rebuild bone.
I used my Forteo as prescribed, in daily injections. The time until my DEXA bone scan passed so slowly. But when the day finally came, the question was: had it been enough time? Had the bone had enough time to grow?
I had my bone scan yesterday. I got the results over the phone today.
This is how to understand a DEXA scan results:
A T-score compares your bone density to the optimal bone density for your gender by standard deviation.
Here's the scale for T-scores:
Osteoporosis: T<-2.5 Osteopenia: T= -2.5 to -1.0 Normal Bone Density: T= -1.0 to 1.0 Increased Bone Density: T> 1.-0
Here are the results of my DEXA scan performed 1/14/2009:
Bone density at the lumbar site: T= -1.8
Bone density at the femoral site: T= -1.3
Here are the results of my DEXA scan performed 2/22/2010:
Bone density at the lumbar site: T= 1.4
Bone density at the femoral site: T= 1.2
This shows a significant improvement after only four months of treatment with Forteo!!!!!
So, I emailed my nurse in New York immediately. I told her my results. I told her to watch the mail for her copy of the results. And I told her that I'm ready any time for the consultation and invasive cervical traction. So, I expect to hear back from her shortly. And I'll keep you up to date.
UPDATE: I LATER FOUND THAT THESE RESULTS WERE INCORRECT AND I WAS MISINFORMED. THE POST-TREATMENT T-VALUES ARE NEGATIVE SCORES.
Unfortunately, this would be a much bigger challenge than I anticipated. My first endocrinologist refused to treat me, because I was too young, and my osteopenia was not severe enough to require treatment. So, I went to a new endocrinologist. She insisted on trying a year of conservative treatment first. I had no choice. She was willing to work with me and my doctors in New York, so she was the best choice I had. The conservative treatment involved calcium supplementation and mega-doses of vitamin D. I ended up with too much vitamin D in my system, but I still wasn't absorbing enough calcium, based on the blood and urine tests. So, on October 26, 2009, she finally prescribed Forteo, which is one of the only medications known to actually rebuild bone.
I used my Forteo as prescribed, in daily injections. The time until my DEXA bone scan passed so slowly. But when the day finally came, the question was: had it been enough time? Had the bone had enough time to grow?
I had my bone scan yesterday. I got the results over the phone today.
This is how to understand a DEXA scan results:
A T-score compares your bone density to the optimal bone density for your gender by standard deviation.
Here's the scale for T-scores:
Osteoporosis: T<-2.5 Osteopenia: T= -2.5 to -1.0 Normal Bone Density: T= -1.0 to 1.0 Increased Bone Density: T> 1.-0
Here are the results of my DEXA scan performed 1/14/2009:
Bone density at the lumbar site: T= -1.8
Bone density at the femoral site: T= -1.3
Here are the results of my DEXA scan performed 2/22/2010:
Bone density at the lumbar site: T= 1.4
Bone density at the femoral site: T= 1.2
This shows a significant improvement after only four months of treatment with Forteo!!!!!
So, I emailed my nurse in New York immediately. I told her my results. I told her to watch the mail for her copy of the results. And I told her that I'm ready any time for the consultation and invasive cervical traction. So, I expect to hear back from her shortly. And I'll keep you up to date.
UPDATE: I LATER FOUND THAT THESE RESULTS WERE INCORRECT AND I WAS MISINFORMED. THE POST-TREATMENT T-VALUES ARE NEGATIVE SCORES.
Sunday, February 7, 2010
An Eventful Day
Friday was quite an eventful day, which is pretty impressive, considering my many limitations.
It started off with a morning in which I couldn't sleep too much. This has continued since then, and I know I'm leaning toward the hypomanic side, so I blame it on this. Due to this, I started reading my book around 8:00, and I got out of bed around 9:15. I ate breakfast. Then, went back upstairs and sorted out some of my stuff (in the a.m. hours). I took a Provigil at this point, because I knew I would need it for the day. (I'm not taking Provigil with any regularity anymore, because it makes me do more than my body is capable of doing.) Next, at about 11:00, I got in the bath, which is actually quite an arduous task, for me. I packed up the rest of my stuff for my weekend in Wisconsin. My mom and I left the house at 1:15 (This is a very active start to the day).
We went to a 1:50 appointment with the eye doctor. I had two scans done there. Since I have very large pupils that are completely non-reactive to light, I didn't need to be dilated for either scan (just another sign of worsening autonomic dysfunction due to brainstem compression). One scan was a 3D optic nerve scan. This was just a couple really bright flashes in each eye. Really bright lights. It took a 3D picture of my optic nerves. Next, I had an optical coherence topography scan done. This one is pretty cool. It shows the depth of the optic nerve. With these scans, they were able to determine that my vision is essentially "safe." Even though the discs are bulging, underneath it, there are thousands of healthy nerve fibers. So, my vision is not at risk, over time, or with surgery. She also explained the bulging as an anatomical anomaly. Whereas most people have optic nerves that are "innies" (think belly buttons), I have "outies." What a perfect explanation, right? So, that's why my optic nerves always look so funny, and seem to perplex so many doctors. I may not have even ever had papilledema in the first place. But my weird optic nerves did catch the needed attention to get a brain mri ordered in the first place. Just another weird anomaly of my very interesting anatomy. I really do have an interesting list of birth defects. What a weird little girl my parents never knew they had!
After the eye doctor, we were hoping to catch a 3:37 train to Milwaukee. Unfortunately, there wasn't nearly enough time to get to the station. So, we had to stall. The next train was at 5:32. It seemed like a lot of time, but the station was a ways away, in rush hour traffic, in snowy weather. So, we decided to stop at Costco to sort out a membership issue. Then, we stopped at Subway to buy my dinner that I would eat on the train. I got to the station by 5:00. I had to sit around a bit, but it's better than missing my train.
Okay, are you ready for another train story?
Well, I was ready for my train this time. I had my bags. I asked for help with only getting my walker up the stairs. Sounds easy enough. I was the last one onto the car, as always, because I'm handicapped. So, I stood there alone, trying to fold up my walker while holding 3 bags. It was actually really hard to do. You need to get the walker at just the right angle to fold the sides in. By the time I had it folded up, I saw that there was absolutely no where to put it. There's only one place on each car to put walkers and wheelchairs, but the area isn't actually reserved for them. It was completely full of suitcases. I decided I would just lean my walker sideways against the suitcases and get to a seat as soon as possible, because I know how hard it can be to find a seat. Unfortunately, I was too late. The train started moving. My walker was already folded up. There was nothing on the walls to hold on to. I fell flat on my butt, and with the movement of the train, rolled backwards onto my back. Everyone in the train car was staring at me, but no one moved to help me. I really didn't know what to do.
Luckily, on my train each weekend, a guy named Marty sells concessions. He is one of the friendliest guys you'll ever meet. While I was on my back, he came running down the aisle. He immediately moved the baggage out of the way for my walker. One lady whined/complained ("Hey! That's my bag!"), but he didn't care at all. Then, he told the person sitting in the front seat she would need to move. She looked shocked and appalled, but she did it immediately. Because Marty meant business. Keep in mind, the train attendant didn't do any of this himself, even though it's his job. Marty helped me up and even brought me a free bottle of water. It's amazing. Everyone will stare at you. But not a single person other than Marty offered to help. They even looked annoyed at having to do anything that might disrupt their day.
So, I should have been set for the rest of my ride, right? No. Because I'm me. But really this part of the story isn't about me, so I will do my best not to make it about me. After about 10 minutes of our ride north, our train came to a sudden stop. The emergency brake and been pulled. I was facing backward (bad idea), so the pressure on my neck of the inertia of the train was intense. The lights flickered off and on. It was a little scary. A very vague announcement was made about an "incident" and a "delay." So, we sat. For quite a while, we were under the impression that something was wrong with the train. Eventually, during one of my phone calls with Gustavo, he read online that pedestrians had been hit by a train in Lake Forest. I knew immediately that it was our train. We learned later that two teenagers were hit, and at least one of them was killed by our train. We stayed where we were for 2 1/2 hours. No passengers were allowed off the train. Marty wasn't his same cheerful self, but he did sell out pretty much all his merchandise, especially all of his alcohol. But we, the passengers, were never officially informed of the nature of the "incident." We figured it out on our own.
My back was in serious pain by the end of the night. The seats recline, but just barely. And my head pain spread throughout my skull. I felt very faint during much of our delay. But again, this night wasn't about me. Some passengers didn't realize that this delay wasn't about them. For example, as we were waiting, one woman was making very loud phone calls during most of the ride. On every phone call, she would say, "I'm having the worst day ever. You wouldn't believe what's happening to me." I understand that it was an uncomfortable inconvenience. But we had a bathroom and concessions that didn't quite run out. So, I'd say, we didn't have it so bad. The families of the victims had a very bad day. The train engineer that left the train with a pale, blank face after having seen everything happen: he had a very bad day. But for the passengers, it couldn't possibly be the worst day.
But, I'm very glad it's over, and I'm very sorry that it happened. It's just a rumor, but I heard that they were just walking across the tracks, and didn't see the lights. There was another death at this same station just a couple months ago.
Moving on, because it's hard to think about this too much.
For me, personally, I need to figure out how to work out the train ride a little bit better. I certainly don't have a manageable system figured out yet. I could go back to the wheelchair, because then I won't need to stand up on the train, but I will have to deal with the lifts and the fact that I don't get a real seat; I have to stay in my wheelchair. That causes the bumps of the ride to be especially rough. So I don't like that option. So, I think what I need to do is the following:
*Get in line with my walker, but collapse the walker on the platform, so I don't have to do it on the train, as it takes up valuable time.
*Ask the attendant to stow the walker for me AND assist me to a seat, so I don't fall down.
*Bring less baggage.
*Wait until the train has definitely stopped to stand up, or I will just fall down again when it lurches at the end.
Maybe, if I do these things, I won't have so many little disasters on the train. I'm still feeling the falls. I really cannot stand up whenever the train is moving. It just multiplies my vertigo, making it impossible to stay upright without strong assistance.
Medication update:
The side effects from the Topamax are wearing off (the tingling), but I don't think my headaches are any better. I will try to increase the dose this week. The Mobic, which is supposed to be like Celebrex, but better, isn't working well at all. I think I'd be better off with Advil, at this point. So, I'm going to test out Celebrex soon.
It started off with a morning in which I couldn't sleep too much. This has continued since then, and I know I'm leaning toward the hypomanic side, so I blame it on this. Due to this, I started reading my book around 8:00, and I got out of bed around 9:15. I ate breakfast. Then, went back upstairs and sorted out some of my stuff (in the a.m. hours). I took a Provigil at this point, because I knew I would need it for the day. (I'm not taking Provigil with any regularity anymore, because it makes me do more than my body is capable of doing.) Next, at about 11:00, I got in the bath, which is actually quite an arduous task, for me. I packed up the rest of my stuff for my weekend in Wisconsin. My mom and I left the house at 1:15 (This is a very active start to the day).
We went to a 1:50 appointment with the eye doctor. I had two scans done there. Since I have very large pupils that are completely non-reactive to light, I didn't need to be dilated for either scan (just another sign of worsening autonomic dysfunction due to brainstem compression). One scan was a 3D optic nerve scan. This was just a couple really bright flashes in each eye. Really bright lights. It took a 3D picture of my optic nerves. Next, I had an optical coherence topography scan done. This one is pretty cool. It shows the depth of the optic nerve. With these scans, they were able to determine that my vision is essentially "safe." Even though the discs are bulging, underneath it, there are thousands of healthy nerve fibers. So, my vision is not at risk, over time, or with surgery. She also explained the bulging as an anatomical anomaly. Whereas most people have optic nerves that are "innies" (think belly buttons), I have "outies." What a perfect explanation, right? So, that's why my optic nerves always look so funny, and seem to perplex so many doctors. I may not have even ever had papilledema in the first place. But my weird optic nerves did catch the needed attention to get a brain mri ordered in the first place. Just another weird anomaly of my very interesting anatomy. I really do have an interesting list of birth defects. What a weird little girl my parents never knew they had!
After the eye doctor, we were hoping to catch a 3:37 train to Milwaukee. Unfortunately, there wasn't nearly enough time to get to the station. So, we had to stall. The next train was at 5:32. It seemed like a lot of time, but the station was a ways away, in rush hour traffic, in snowy weather. So, we decided to stop at Costco to sort out a membership issue. Then, we stopped at Subway to buy my dinner that I would eat on the train. I got to the station by 5:00. I had to sit around a bit, but it's better than missing my train.
Okay, are you ready for another train story?
Well, I was ready for my train this time. I had my bags. I asked for help with only getting my walker up the stairs. Sounds easy enough. I was the last one onto the car, as always, because I'm handicapped. So, I stood there alone, trying to fold up my walker while holding 3 bags. It was actually really hard to do. You need to get the walker at just the right angle to fold the sides in. By the time I had it folded up, I saw that there was absolutely no where to put it. There's only one place on each car to put walkers and wheelchairs, but the area isn't actually reserved for them. It was completely full of suitcases. I decided I would just lean my walker sideways against the suitcases and get to a seat as soon as possible, because I know how hard it can be to find a seat. Unfortunately, I was too late. The train started moving. My walker was already folded up. There was nothing on the walls to hold on to. I fell flat on my butt, and with the movement of the train, rolled backwards onto my back. Everyone in the train car was staring at me, but no one moved to help me. I really didn't know what to do.
Luckily, on my train each weekend, a guy named Marty sells concessions. He is one of the friendliest guys you'll ever meet. While I was on my back, he came running down the aisle. He immediately moved the baggage out of the way for my walker. One lady whined/complained ("Hey! That's my bag!"), but he didn't care at all. Then, he told the person sitting in the front seat she would need to move. She looked shocked and appalled, but she did it immediately. Because Marty meant business. Keep in mind, the train attendant didn't do any of this himself, even though it's his job. Marty helped me up and even brought me a free bottle of water. It's amazing. Everyone will stare at you. But not a single person other than Marty offered to help. They even looked annoyed at having to do anything that might disrupt their day.
So, I should have been set for the rest of my ride, right? No. Because I'm me. But really this part of the story isn't about me, so I will do my best not to make it about me. After about 10 minutes of our ride north, our train came to a sudden stop. The emergency brake and been pulled. I was facing backward (bad idea), so the pressure on my neck of the inertia of the train was intense. The lights flickered off and on. It was a little scary. A very vague announcement was made about an "incident" and a "delay." So, we sat. For quite a while, we were under the impression that something was wrong with the train. Eventually, during one of my phone calls with Gustavo, he read online that pedestrians had been hit by a train in Lake Forest. I knew immediately that it was our train. We learned later that two teenagers were hit, and at least one of them was killed by our train. We stayed where we were for 2 1/2 hours. No passengers were allowed off the train. Marty wasn't his same cheerful self, but he did sell out pretty much all his merchandise, especially all of his alcohol. But we, the passengers, were never officially informed of the nature of the "incident." We figured it out on our own.
My back was in serious pain by the end of the night. The seats recline, but just barely. And my head pain spread throughout my skull. I felt very faint during much of our delay. But again, this night wasn't about me. Some passengers didn't realize that this delay wasn't about them. For example, as we were waiting, one woman was making very loud phone calls during most of the ride. On every phone call, she would say, "I'm having the worst day ever. You wouldn't believe what's happening to me." I understand that it was an uncomfortable inconvenience. But we had a bathroom and concessions that didn't quite run out. So, I'd say, we didn't have it so bad. The families of the victims had a very bad day. The train engineer that left the train with a pale, blank face after having seen everything happen: he had a very bad day. But for the passengers, it couldn't possibly be the worst day.
But, I'm very glad it's over, and I'm very sorry that it happened. It's just a rumor, but I heard that they were just walking across the tracks, and didn't see the lights. There was another death at this same station just a couple months ago.
Moving on, because it's hard to think about this too much.
For me, personally, I need to figure out how to work out the train ride a little bit better. I certainly don't have a manageable system figured out yet. I could go back to the wheelchair, because then I won't need to stand up on the train, but I will have to deal with the lifts and the fact that I don't get a real seat; I have to stay in my wheelchair. That causes the bumps of the ride to be especially rough. So I don't like that option. So, I think what I need to do is the following:
*Get in line with my walker, but collapse the walker on the platform, so I don't have to do it on the train, as it takes up valuable time.
*Ask the attendant to stow the walker for me AND assist me to a seat, so I don't fall down.
*Bring less baggage.
*Wait until the train has definitely stopped to stand up, or I will just fall down again when it lurches at the end.
Maybe, if I do these things, I won't have so many little disasters on the train. I'm still feeling the falls. I really cannot stand up whenever the train is moving. It just multiplies my vertigo, making it impossible to stay upright without strong assistance.
Medication update:
The side effects from the Topamax are wearing off (the tingling), but I don't think my headaches are any better. I will try to increase the dose this week. The Mobic, which is supposed to be like Celebrex, but better, isn't working well at all. I think I'd be better off with Advil, at this point. So, I'm going to test out Celebrex soon.
Wednesday, February 3, 2010
Coming out of hibernation
So, I haven't written in quite a while, because my life has been stuck at such a standstill. Lying around in bed all day doesn't provide much inspiration for writing. I think I've finally collected enough little stories to add up to one post.
I've seen Dr. W, my pain management doctor, a few times. I've been taking Topamax for my chronic daily migraine. Unfortunately, I can't get up to a very high dose, because of my main side effect. The problem I have is with tingling in my hands, feet, and face. So, I'm not sure how much that medication will be able to help, unless the side effect wears off. Also, I've been put on Mobic. It is for arthritis pain. I've been on it for two days, so it's early, but I think it helps a little. I took my mom's Celebrex one day, out of desperation, and it seemed to help much more. But I'll give my medication a fair chance, before I ask for a switch.
I'm having severe hayfever symptoms, even though my last allergy tests were negative. My medications are not helping, and the symptoms are quite severe at times. The sneezing is definitely making my headache worse. Same with the sinus problems. I'll be seeing the allergist tomorrow.
I'm seeing the eye doctor for a 3D optic nerve scan Friday.
I also started seeing a new psychiatrist. Over the holidays, it became clear that my psychological state was evolving. I didn't feel comfortable going to my current doctor with this issue. So, I arranged to see a new psychiatrist in January. I really like her a lot, and I really trust my treatment to her. It's hard to say whether my psychological condition is organic (occurring naturally) or secondary to a neurological or pharmacological problem. Nonetheless, it needs to be treated and managed. I have developed symptoms of hypomania that are very troublesome at times. This is a symptom of a condition called Bipolar II. That is the "lesser" form of Bipolar, because the episodes of mania are less severe. However, the episodes of depression are no less severe. This is not necessarily part of the Bipolar II. As long as I stay on top of my meds, I don't have much trouble with it, for now, thank goodness. This is actually a manageable condition, unlike my others. I should mention, though, for some people, it is much less manageable. I've just been very lucky, so far. In addition, I have a condition involving rage episodes. This is a very difficult thing to deal with, and can be quite severe at times.
We removed the car insurance from my car, because I haven't driven it in so long. That's perfectly logical. It just makes it very clear that I will not be well enough to drive for a very long time. It's an overwhelming thought: that I will be trapped in this house most of the time for so long. And that I really have lost my independence. I can't go anywhere without asking someone else first!!!! This is a tough one to get used to. But it's made less hard by the fact that it's been a very gradual transition. I actually haven't driven my car in at least two months, so this isn't actually something new. It's just the possibility that is gone.
Luckily, with the money we're saving on car insurance, we were able to buy a 10-ride ticket on the Amtrak for me to go to Milwaukee. So I'll be able to take the train into Milwaukee to be with Gustavo most weekends in the near future. It's quite an expensive ride, and the ride to the station is a little out of the way on both ends, but it's worth it to continue my weekend visits.
So far, I've taken the train twice. The first time, I used my wheelchair, and bought a handicapped ticket (which gets you 15% off, with an unpublished discount). When you want to ride the train in a wheelchair, you have to call ahead, and arrive early. Then, you have to ride out to the train, with all your stuff in your lap. Then, you roll onto a lift. They crank the lift up. Next, you have to ride off the lift, onto the train. You can tell that they don't do it very often, because they're not very good at getting the levels even, or lined up with the train. It seems like a pretty perilous transfer. Luckily, I made it okay onto the train. There's only one place you can sit. It's next to the garbage cans in the front of the car. You slide into place facing sideways; then lock into place. You don't get to move the entire ride. You hold your baggage on your lap the entire ride. I was incredibly uncomfortable. Also, while the other passengers are casually swaying side to side, you are in your own miniature amusement park ride, getting miniature cases of whiplash every time the train "sways" from side to side. Since you are facing sideways, this sway is a front-to-back jerking to you. Overall, the ride was incredibly uncomfortable, and I wouldn't recommend sideways train riding to anyone with neck damage. Also, the luggage in my lap made it impossible to readjust my position or stretch my legs. So my legs and back were all knotted up by the end of the ride. I couldn't believe the passengers so near to me were yawning, listening to iPods, casually relaxing, or even sleeping through this nightmare!
Then, on the ride home, my fellow train-riders and I were in for a surprise, as we waited an hour and a half for our train to be ready to board. When the announcement was finally made, I wasn't too surprised to hear that the train was not in working order, and we would all be taking buses to Chicago. This was all new to me, but I figured if I could survive the ride in on the train, I could certainly survive the ride out on a bus. But that was not the plan. Since I was their only "special" passenger, I was the only one that would not be taking the buses, which were not accessible. Instead, I would be taking a cab all the way to Chicago. It was already 10:00 at night. If I were given the choice, I probably would have chosen to stay in Milwaukee one more day, but this was already planned without me. So, I waited another 25 minutes, until after the buses had already departed. My "cab" came. It was actually just a guy in a car. Not even a specially marked car. Luckily, he was legitimate. But my wheelchair had to be completely broken down to fit in his car. This was definitely not any more accessible than the bus would have been. Plus, we ran out of gas, and he didn't have money. Plus, he didn't use any heat, in January. So it was freeeeeezing. And I had such a headache after the long night, so the loud music didn't help.
So, after taking the train on a wheelchair, you'd think I wouldn't be anxious to do it again. But when you run out of transportation options, public transportation starts to look pretty good. So, the next time, I decided that maybe the train would work better if I took my walker. At least I wouldn't need the lift, right? So, we bought the 10-ride ticket, which gives you a 25% discount. You can't get a discount for being disabled, in addition. So this discount is better, anyway. When my train arrived, I was zoning out. I didn't even hear it called. So the guy working at the station came to help me, saying he didn't want me to miss my train, which was really nice. He offered to help me with my stuff, which was also really nice. I had with me: my walker, a messenger bag, a backpack, and a purse. He took: the walker and the messenger bag. Yes. The walker. He pushed the walker all the way out to the platform from inside the station. Luckily, I can walk without the walker. I use it for security, for balance, and for sitting. But this was one of the strangest kinds of help I've ever received. At this point, a worker on the train took the walker from him and loaded it on the train. I had to walk all by myself from the station to the platform and onto the train. My messenger bag was dropped onto the train for me. Unfortunately, it had my laptop in it, when it was dropped. This seemed like a very strange way of helping me. So, I was very tired at this point. I was out of breath and my legs were starting to give out on me. I had to find a seat, and none were readily available. I walked the entire length of the car (about 30 rows), carrying my 3 bags, holding onto seats as I walked. Whenever I saw an empty seat, it was a window seat, and the person in the aisle seat, blocking it, was either asleep, or pretending to sleep. I reached the other end of the car, and still had no seat. By this point, I was very weak, tired, and woozy.
Plus, the train was moving by now, so my vertigo was worse than usual. I could barely stay upright, and probably appeared drunk. What choice did I have but to turn around, looking even more pathetic, and this time, actually start begging for a seat. Luckily, the first person I asked actually took pity on me and let me sit. Probably because I looked quite sickly at this point. Once I sat down, I could finally relax. Other than my problem with germs in public places, I found myself to be pretty comfortable. My seat reclined (heaven). I didn't even need something to do. The crazy "turbulence" didn't bother me too much. Of course, I still wore my neckbrace, which I always wear. But, the side-to-side swaying didn't seem so rough. When I reached my stop, I took charge of my own possessions, meaning I carried my three bags and my walker down the steps (very difficult), but it was worth it. My ride back to Chicago was actually uneventful. No one stole my walker, even for a short time. I had temporary help with my bags. And I got a seat in the front row. So easy.
My other news is that I have an appointment with Dr. F, geneticist extraordinaire, scheduled! I'll be seeing her February 17th. I've been trying to figure out what to expect from this appointment, and what to ask her. I always compile a complete list of concerns and questions before an important doctor's appointment. I'm just not sure which concerns should be directed toward her. I know that she specializes in EDS. But she also helps people with POTS. And she is the one to talk to with genetic counseling type questions. So here's what I've got so far:
1. a. What can help with the especially slow recovery after cranio-cervical fusion (and revision)? My recovery took at least two years last time, with overwhelming and lasting fatigue, paralyzing muscle spasms, and relentless pain.
b. What kind of improvement is reasonable to expect (long-term) from a cranio-cervical fusion?
2. a. How to make a physical therapist understand EDS and POTS and establish a helpful and safe exercise routine?
b. What kinds of exercise are helpful and safe?
c. What movements/activities should be avoided to keep my joints strong as long as possible?
3. a. Is it okay to be on medication like Mobic/Celebrex/Vicodin forever?
4. a. What are my chances of passing this on (Chiari/EDS/POTS)--even a reasonable guess would be appreciated; any input would be appreciated?
5. a. Are there any vitamins/supplements that I should be taking regularly?
b. Is there a way to test to find out my levels of important vitamins to check for deficiencies?
6. a. I take 2000+ mg NaCl/day to maintain consciousness, but I'm worried about my bones. Any ideas on what else I can do about this?
b. I am on Forteo, to strengthen my bones before surgery, but I know my endocrinologist won't let me stay on it post-surgically, because she doesn't believe in off-label use. What should I do after that to keep my bone density up?
I have a million other questions, but I don't know if they're really her "field" or not. I can't just ask her every question that I don't know who else to ask. But I am also wondering about the following things:
*Heart palpitations-strong and resonating (intermittent symptom)
*hypersensitivity in skin (intermittent)
*severe PMDD, hormonal problems--------maybe this one
*IBS/bloating/severe abdominal pain (intermittent)--------maybe this one
*severe allergies with negative allergy testing (intermittent)
*Glucose level high in every blood test, but not diabetic--------maybe
I've seen Dr. W, my pain management doctor, a few times. I've been taking Topamax for my chronic daily migraine. Unfortunately, I can't get up to a very high dose, because of my main side effect. The problem I have is with tingling in my hands, feet, and face. So, I'm not sure how much that medication will be able to help, unless the side effect wears off. Also, I've been put on Mobic. It is for arthritis pain. I've been on it for two days, so it's early, but I think it helps a little. I took my mom's Celebrex one day, out of desperation, and it seemed to help much more. But I'll give my medication a fair chance, before I ask for a switch.
I'm having severe hayfever symptoms, even though my last allergy tests were negative. My medications are not helping, and the symptoms are quite severe at times. The sneezing is definitely making my headache worse. Same with the sinus problems. I'll be seeing the allergist tomorrow.
I'm seeing the eye doctor for a 3D optic nerve scan Friday.
I also started seeing a new psychiatrist. Over the holidays, it became clear that my psychological state was evolving. I didn't feel comfortable going to my current doctor with this issue. So, I arranged to see a new psychiatrist in January. I really like her a lot, and I really trust my treatment to her. It's hard to say whether my psychological condition is organic (occurring naturally) or secondary to a neurological or pharmacological problem. Nonetheless, it needs to be treated and managed. I have developed symptoms of hypomania that are very troublesome at times. This is a symptom of a condition called Bipolar II. That is the "lesser" form of Bipolar, because the episodes of mania are less severe. However, the episodes of depression are no less severe. This is not necessarily part of the Bipolar II. As long as I stay on top of my meds, I don't have much trouble with it, for now, thank goodness. This is actually a manageable condition, unlike my others. I should mention, though, for some people, it is much less manageable. I've just been very lucky, so far. In addition, I have a condition involving rage episodes. This is a very difficult thing to deal with, and can be quite severe at times.
We removed the car insurance from my car, because I haven't driven it in so long. That's perfectly logical. It just makes it very clear that I will not be well enough to drive for a very long time. It's an overwhelming thought: that I will be trapped in this house most of the time for so long. And that I really have lost my independence. I can't go anywhere without asking someone else first!!!! This is a tough one to get used to. But it's made less hard by the fact that it's been a very gradual transition. I actually haven't driven my car in at least two months, so this isn't actually something new. It's just the possibility that is gone.
Luckily, with the money we're saving on car insurance, we were able to buy a 10-ride ticket on the Amtrak for me to go to Milwaukee. So I'll be able to take the train into Milwaukee to be with Gustavo most weekends in the near future. It's quite an expensive ride, and the ride to the station is a little out of the way on both ends, but it's worth it to continue my weekend visits.
So far, I've taken the train twice. The first time, I used my wheelchair, and bought a handicapped ticket (which gets you 15% off, with an unpublished discount). When you want to ride the train in a wheelchair, you have to call ahead, and arrive early. Then, you have to ride out to the train, with all your stuff in your lap. Then, you roll onto a lift. They crank the lift up. Next, you have to ride off the lift, onto the train. You can tell that they don't do it very often, because they're not very good at getting the levels even, or lined up with the train. It seems like a pretty perilous transfer. Luckily, I made it okay onto the train. There's only one place you can sit. It's next to the garbage cans in the front of the car. You slide into place facing sideways; then lock into place. You don't get to move the entire ride. You hold your baggage on your lap the entire ride. I was incredibly uncomfortable. Also, while the other passengers are casually swaying side to side, you are in your own miniature amusement park ride, getting miniature cases of whiplash every time the train "sways" from side to side. Since you are facing sideways, this sway is a front-to-back jerking to you. Overall, the ride was incredibly uncomfortable, and I wouldn't recommend sideways train riding to anyone with neck damage. Also, the luggage in my lap made it impossible to readjust my position or stretch my legs. So my legs and back were all knotted up by the end of the ride. I couldn't believe the passengers so near to me were yawning, listening to iPods, casually relaxing, or even sleeping through this nightmare!
Then, on the ride home, my fellow train-riders and I were in for a surprise, as we waited an hour and a half for our train to be ready to board. When the announcement was finally made, I wasn't too surprised to hear that the train was not in working order, and we would all be taking buses to Chicago. This was all new to me, but I figured if I could survive the ride in on the train, I could certainly survive the ride out on a bus. But that was not the plan. Since I was their only "special" passenger, I was the only one that would not be taking the buses, which were not accessible. Instead, I would be taking a cab all the way to Chicago. It was already 10:00 at night. If I were given the choice, I probably would have chosen to stay in Milwaukee one more day, but this was already planned without me. So, I waited another 25 minutes, until after the buses had already departed. My "cab" came. It was actually just a guy in a car. Not even a specially marked car. Luckily, he was legitimate. But my wheelchair had to be completely broken down to fit in his car. This was definitely not any more accessible than the bus would have been. Plus, we ran out of gas, and he didn't have money. Plus, he didn't use any heat, in January. So it was freeeeeezing. And I had such a headache after the long night, so the loud music didn't help.
So, after taking the train on a wheelchair, you'd think I wouldn't be anxious to do it again. But when you run out of transportation options, public transportation starts to look pretty good. So, the next time, I decided that maybe the train would work better if I took my walker. At least I wouldn't need the lift, right? So, we bought the 10-ride ticket, which gives you a 25% discount. You can't get a discount for being disabled, in addition. So this discount is better, anyway. When my train arrived, I was zoning out. I didn't even hear it called. So the guy working at the station came to help me, saying he didn't want me to miss my train, which was really nice. He offered to help me with my stuff, which was also really nice. I had with me: my walker, a messenger bag, a backpack, and a purse. He took: the walker and the messenger bag. Yes. The walker. He pushed the walker all the way out to the platform from inside the station. Luckily, I can walk without the walker. I use it for security, for balance, and for sitting. But this was one of the strangest kinds of help I've ever received. At this point, a worker on the train took the walker from him and loaded it on the train. I had to walk all by myself from the station to the platform and onto the train. My messenger bag was dropped onto the train for me. Unfortunately, it had my laptop in it, when it was dropped. This seemed like a very strange way of helping me. So, I was very tired at this point. I was out of breath and my legs were starting to give out on me. I had to find a seat, and none were readily available. I walked the entire length of the car (about 30 rows), carrying my 3 bags, holding onto seats as I walked. Whenever I saw an empty seat, it was a window seat, and the person in the aisle seat, blocking it, was either asleep, or pretending to sleep. I reached the other end of the car, and still had no seat. By this point, I was very weak, tired, and woozy.
Plus, the train was moving by now, so my vertigo was worse than usual. I could barely stay upright, and probably appeared drunk. What choice did I have but to turn around, looking even more pathetic, and this time, actually start begging for a seat. Luckily, the first person I asked actually took pity on me and let me sit. Probably because I looked quite sickly at this point. Once I sat down, I could finally relax. Other than my problem with germs in public places, I found myself to be pretty comfortable. My seat reclined (heaven). I didn't even need something to do. The crazy "turbulence" didn't bother me too much. Of course, I still wore my neckbrace, which I always wear. But, the side-to-side swaying didn't seem so rough. When I reached my stop, I took charge of my own possessions, meaning I carried my three bags and my walker down the steps (very difficult), but it was worth it. My ride back to Chicago was actually uneventful. No one stole my walker, even for a short time. I had temporary help with my bags. And I got a seat in the front row. So easy.
My other news is that I have an appointment with Dr. F, geneticist extraordinaire, scheduled! I'll be seeing her February 17th. I've been trying to figure out what to expect from this appointment, and what to ask her. I always compile a complete list of concerns and questions before an important doctor's appointment. I'm just not sure which concerns should be directed toward her. I know that she specializes in EDS. But she also helps people with POTS. And she is the one to talk to with genetic counseling type questions. So here's what I've got so far:
1. a. What can help with the especially slow recovery after cranio-cervical fusion (and revision)? My recovery took at least two years last time, with overwhelming and lasting fatigue, paralyzing muscle spasms, and relentless pain.
b. What kind of improvement is reasonable to expect (long-term) from a cranio-cervical fusion?
2. a. How to make a physical therapist understand EDS and POTS and establish a helpful and safe exercise routine?
b. What kinds of exercise are helpful and safe?
c. What movements/activities should be avoided to keep my joints strong as long as possible?
3. a. Is it okay to be on medication like Mobic/Celebrex/Vicodin forever?
4. a. What are my chances of passing this on (Chiari/EDS/POTS)--even a reasonable guess would be appreciated; any input would be appreciated?
5. a. Are there any vitamins/supplements that I should be taking regularly?
b. Is there a way to test to find out my levels of important vitamins to check for deficiencies?
6. a. I take 2000+ mg NaCl/day to maintain consciousness, but I'm worried about my bones. Any ideas on what else I can do about this?
b. I am on Forteo, to strengthen my bones before surgery, but I know my endocrinologist won't let me stay on it post-surgically, because she doesn't believe in off-label use. What should I do after that to keep my bone density up?
I have a million other questions, but I don't know if they're really her "field" or not. I can't just ask her every question that I don't know who else to ask. But I am also wondering about the following things:
*Heart palpitations-strong and resonating (intermittent symptom)
*hypersensitivity in skin (intermittent)
*severe PMDD, hormonal problems--------maybe this one
*IBS/bloating/severe abdominal pain (intermittent)--------maybe this one
*severe allergies with negative allergy testing (intermittent)
*Glucose level high in every blood test, but not diabetic--------maybe
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